Combined life-threatening thromboses and hemorrhages in a patient with afibrinogenemia and antithrombin deficiency.

BACKGROUND: Patients with congenital afibrinogenemia suffer from spontaneous recurrent severe bleeding. While fibrinogen concentrates are known to effectively treat bleeding episodes, thrombotic complications often occur upon replacement therapy, rendering clinical management highly challenging. CASE PRESENTATION: We hereby report a case of combined afibrinogenemia and congenital antithrombin deficiency manifested by recurrent life-threatening bleeding, as well as spontaneous severe arterial occlusion, such as acute coronary syndrome and stroke, and venous thromboses like pulmonary embolism.Secondary fibrinogen prophylaxis is recommended following any initial life-threatening bleeding episode in patients with afibrinogenemia, yet the high associated risk of thrombosis illustrates the complexity of choosing the most effective prophylaxis strategy combining fibrinogen concentrate with antithrombotic agent for optimal protection against the risk of both severe bleeding and thrombosis. For our patient, the thrombin generation assay objectively confirmed her prothrombotic tendency. CONCLUSION: This case may help us better understand the pathophysiology of arterial thrombosis in afibrinogenemia, while highlighting the difficulty of managing such complications.

New parameters on the hematology analyzer XN-10 (SysmexTM) allow to distinguish childhood bacterial and viral infections.

INTRODUCTION: Complete blood counts (CBC) performed for infected children admitted for fever mostly disclose leukocytosis. Yet, the recently developed XN-10® provides novel CBC parameters which could be useful to ascertain infection and discriminate between bacterial and viral etiologies. These were the main objectives of the study presented here. METHODS: Blood samples from 90 children, 1 month to 5 years old, admitted to an emergency unit for fever benefited from a CBC, C-reactive protein, and procalcitonin assays. For 58, a bacterial infection was documented while a viral cause was disclosed for 32. Concomitantly, 30 healthy children of the same age range were selected as a control group. RESULTS: Complete blood counts parameters and leukocyte differentials allowed to statistically significantly disclose infection, compared to reference children, in the age group of 1-5 years old. Among the eight novel discriminant parameters, a particular interest appeared for Neutr-RI and Delta-He. They both were successfully incorporated in a score together with age and immature granulocytes (IG). ROC curves and AUCs were calibrated using a Hosmer-Lemeshow test. Moreover, novel lymphocyte parameters allowed to segregate bacterial and viral infections in the whole group of 90 febrile children. CONCLUSION: Complete blood counts is the most broadly performed rapid laboratory investigation. Here, we show that XN-10® provides complementary information allowing to confirm infection in febrile children, moreover discriminating between bacterial or viral origin.

Acute lymphoblastic leukemia relapsing after first-line pediatric-inspired therapy: a retrospective GRAALL study.

The outcome of adult patients with Philadelphia chromosome-negative acute lymphoblastic leukemia (Ph- ALL) relapsing after pediatric-inspired front-line therapy is ill known. Here 229 relapsing Ph- ALL younger adults (18-63 years) treated within the Group for Research on Adult Acute Lymphoblastic Leukemia (GRAALL)-2003/-2005 trials were considered. Salvage regimens consisted of potentially curative therapies in 194 cases, low-intensity therapies in 21, allogeneic stem cell transplant (allo-SCT) in 6 and best supportive care in 8. Overall, 77 patients received allo-SCT after relapse. The median follow-up was 3.1 years. A second complete remission (CR2) was achieved in 121 patients (53%). In multivariate analysis, only younger age <45 years (P=0.008) and CR1 duration ⩾18 months (P=0.009) predicted CR2. Overall survival (OS) at 2 and 5 years was 19.3% (14-24%) and 13.3% (8-18%), respectively. In CR2 patients, disease-free survival (DFS) at 2 and 5 years was 29.0% (21-38%) and 25% (17-33%). In multivariate analysis, CR1 duration ⩾18 months and allo-SCT after relapse were associated with longer DFS (P<0.009 and P=0.004, respectively) and longer OS (P=0.004 and P<0.0001, respectively). In conclusion, although younger adults relapsing after pediatric-inspired ALL therapies retain a poor outcome, some of them may be cured if CR1 duration ⩾18 months and if allo-SCT can be performed in CR2. New therapies are definitely needed for these patients.